[Surgical treatment of mega aorta syndrome: a single-center experience].

2021 
OBJECTIVE To present our experience in the treatment of severe patients with mega aorta syndrome. MATERIAL AND METHODS There were 49 patients with mega aorta syndrome for the period from May 2015 to March 2021. All patients underwent total aortic replacement from sinotubular junction (with staged aortic root repair, if necessary) to abdominal aorta bifurcation. All surgeries were elective. All patients were divided into two groups: group I (n=33; 67.3%) - staged replacement, group II (n=16; 32.7%) - one-staged replacement of the aorta via thoracophrenolumbotomy. The primary endpoints were mortality, perioperative parameters and complications. We also analyzed long-term freedom from aortic redo surgery and survival rate. RESULTS Both groups were comparable by pre-, intra- and postoperative parameters. The interval between surgeries for staged approach was 7.1±2.3 months. Rehabilitation time considering two stages was longer in group I (13±2 vs. 5.5±1.1 months, p=0.0001). Between-stage mortality rate was 12% (n=4). Intraoperative mortality was absent in both groups. In-hospital mortality was 3% and 12% (p=0.25), overall mortality with between-stage interval - 10.2% and 12% (p=1.000), respectively. The follow-up period was similar (18±22.7 (range 1-71) and 23.3±19.1 (range 1-51) months, respectively (p=0.63)). In group I, 1-year, 3-year and 5-year survival rate considering between-stage mortality was 89% (95% CI 78-100%), 77.1% (95% CI 60.1-98.8%), 77.1% (95% CI 60.1-98.8%), respectively. In group II, 1-year and 3-year survival rate was 86.5% (95% CI 70.5-100%), plog-rank=0.88. Overall freedom from redo surgery was 92.9% (95% CI 80.3-100%) vs. 90.9% (95% CI 75.4-100%), plog-rank=0.072. CONCLUSION One-stage total aortic replacement via thoracophrenolumbotomy is safe surgical treatment for mega aorta syndrome, especially in young patients with low surgical risk. Favorable outcomes may be expected in specialized centers with extensive experience in aortic surgery. Compared to staged approach, total aortic replacement eliminates the risks of between-stage aortic rupture. Therefore, it is a worthy alternative to other methods.
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